D2.129 - Cardiac onset of EGPA in a postpartum woman: the importance of early diagnosis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare necrotizing systemic vasculitis affecting small- and medium-sized vessels. It may involve multiple organs, with asthma being a hallmark feature. Cardiac involvement is uncommon but represents one of the most severe and potentially life-threatening manifestations, making early diagnosis and treatment essential.

We report the case of a 32-year-old woman with allergic rhinitis and pollen sensitization who developed recurrent wheezing, dyspnea and sinusitis after her first pregnancy in 2023.

 In September 2025, one month after her second delivery, she was admitted for recurrent epigastric pain, diffuse abdominal pain and diarrhea associated with peripheral eosinophilia, and was discharged with a diagnosis of nonspecific gastroenteritis. During this admission she developed an anaphylactic reaction after ketorolac administration.

In November 2025, she was readmitted for oppressive chest pain radiating to the upper limbs, without ventricular dysfunction, and was diagnosed with acute myocarditis, again associated with eosinophilia. Due to suspected EGPA, cyclophosphamide was proposed but declined because of breastfeeding. She received intravenous methylprednisolone (500 mg/day for three days) and a single dose of intravenous rituximab (1 g), with subsequent clinical improvement. No renal, cutaneous or neurological involvement was detected.

Peripheral eosinophilia fluctuated (>500/µL), reaching a peak of 2680/µL in September 2025. (Figure 1)

Abdominal CT and stool cultures were normal; celiac serology was negative; upper endoscopy with biopsies showed no eosinophilic infiltration.

 Thoracic CT revealed diffuse bronchial wall thickening and basal right ground-glass opacities compatible with subclinical bronchiolitis. In November, troponin levels were elevated (~300 ng/L) without ventricular dysfunction. Cardiac MRI demonstrated myocardial edema with prolonged T1/T2 values and no late gadolinium enhancement, consistent with acute myocarditis. ANCA were negative. CRP and serum amyloid A remained normal.

 Spirometry was normal FEV1 3.28L (98%), FVC 4.04L (101%), FEV1/FVC 81.37%, FEF 25-75% 3.17L (88%).

EGPA should be considered in patients with asthma, eosinophilia and systemic involvement. In ANCA-negative disease, cardiac involvement may represent the true vasculitic onset following prior asthmatic and eosinophilic phases. ANCA stratification helps to interpret clinical phenotype and target organs. In breastfeeding patients, rituximab represents a valid therapeutic alternative when cytotoxic agents are contraindicated, highlighting the importance of multidisciplinary management for early diagnosis and treatment.